Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment

Abstract

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure plus cerebellar syndrome and/or parkinsonism. Dysphagia is a frequent and disabling symptom in MSA and its occurrence within 5 years of motor onset is an additional diagnostic feature. Dysphagia can lead to aspiration pneumonia, a recognized cause of death in MSA. Guidelines for diagnosis and management of dysphagia in MSA are lacking. An International Consensus Conference among experts with methodological support was convened in Bologna to reach consensus statements for the diagnosis, prognosis, and treatment of dysphagia in MSA. Abnormalities of the oral and pharyngeal phases of swallowing, esophageal dysfunction and aspiration occur in MSA and worsen as the disease progresses. According to the consensus, dysphagia should be investigated through available screening questionnaires and clinical and instrumental assessment (videofluoroscopic study or fiberoptic endoscopic evaluation of swallowing and manometry) at the time of MSA diagnosis and periodically thereafter. There is evidence that dysphagia is associated with poor survival in MSA, however effective treatments for dysphagia are lacking. Compensatory strategies like diet modification, swallowing maneuvers and head postures should be applied and botulinum toxin injection may be effective in specific conditions. Percutaneous endoscopic gastrostomy may be performed when there is a severe risk of malnutrition and pulmonary complications, but its impact on survival is undetermined. Several research gaps and unmet needs for research involving diagnosis, prognosis, and treatment were identified.

Improving the Accuracy of Diagnosis for Multiple-System Atrophy Using Deep Learning-Based Method

“…The higher frequency of cases assigned to this subtype compared to the other subtypes may be due to these specific findings. It is of note that dysphagia was identified as an important feature for SND because this condition is an additional feature of possible MSA-P and a poor prognostic factor for MSA-P [ 29 ].…”

A Systematic Review of Oropharyngeal Dysphagia Models in Rodents

“…Any of these issues can result in penetration/aspiration and can also impact the esophageal phase [ 50 ]. Oropharyngeal dysphagia is generally attributed to neurological causes that lead to injuries (e.g., ischemic, traumatic, neoplastic, neurodegenerative, and infectious) in the nervous system that initiate and control swallowing (e.g., basal ganglia, brain stem, cerebellum, and cranial nerve) [ 50 , 51 , 52 ]. Neurologic diseases include strokes, brain tumors, movement disorders such as PD and HD, MS, cognitive impairments, and motor neuron diseases of the cerebellum and the brain stem such as ALS.…”

Neuropathology, Genetics and Biomarkers of Synucleinopathies

“…Autonomic failure, such as urinary incontinence and orthostatic hypotension, is required for the clinical diagnosis of MSA. Besides autonomic failure, non-motor symptoms, such as RBD, sleep-disordered breathing, dysphagia, and severe dysphonia and dysarthria, are also common in MSA [33][34][35]. Unlike PDD and DLB, dementia is not a typical feature of MSA [33]; however, a subset of patients develops cognitive de cits, particularly executive dysfunction [36][37][38].…”